Psychogenic Non-Epileptic Seizures

Purpose of Review

In this review, we elucidate the evaluation process involved in the diagnosis of psychogenic non-epileptic seizures (PNES). Minimum clinical criteria required to attain this diagnosis via a staged approach are delineated. The psychological underpinnings and management of PNES from the neurologists’ perspective are also explored.

Recent Findings

Helpful clues can be deduced from history-taking, seizure semiology, ictal/peri-ictal physical exam, and ictal/inter-ictal EEG data. No single clinical data point is definitively diagnostic of PNES. Instead, the level of certainty for PNES diagnosis is contingent upon concordance of the composite clinical evidence available. Robust neurologist-patient alliance not only facilitates the evaluation process but can influence therapeutic impact.

Summary

While diagnosis of PNES can be challenging, this diagnosis can be reliably made upon establishing concordance of the historical, physical exam, and video-EEG findings. Evidence-based treatments are available for patients with PNES. Continued efforts remain necessary to enhance timely diagnosis and interdisciplinary management for patients with PNES.

     

Detection of fast (40–150 Hz) oscillations from the ictal scalp EEG data of myoclonic seizures in pediatric patients

Objective

We explored fast (40–150?Hz) oscillations (FOs) from the ictal scalp electroencephalogram (EEG) data of myoclonic seizures in pediatric patients to obtain insight into the pathophysiological mechanisms involved in the generation of myoclonic seizures.

Analysis of ictal magnetoencephalography using gradient magnetic-field topography (GMFT) in patients with neocortical epilepsy

Objective

We aimed to validate the usefulness of gradient magnetic-field topography (GMFT) for analysis of ictal magnetoencephalography (MEG) in patients with neocortical epilepsy.

Magnetoencephalography and ictal SPECT in patients with failed epilepsy surgery

Objective

Selected patients with intractable focal epilepsy who have failed a previous epilepsy surgery can become seizure-free with reoperation. Preoperative evaluation is exceedingly challenging in this cohort. We aim to investigate the diagnostic value of two noninvasive approaches, magnetoencephalography (MEG) and ictal single-photon emission computed tomography (SPECT), in patients with failed epilepsy surgery.

SCOPE-mTL: A non-invasive tool for identifying and lateralizing mesial temporal lobe seizures prior to scalp EEG ictal onset

Objective

In mesial temporal lobe (mTL) epilepsy, seizure onset can precede the appearance of a scalp EEG ictal pattern by many seconds. The ability to identify this early, occult mTL seizure activity could improve lateralization and localization of mTL seizures on scalp EEG.

Clinico – diagnostic and therapeutic relevance of computed tomography scan of brain in children with partial seizures

Background:

Therapeutic relevance of computed tomography (CT) in children with partial seizures is reported to be remarkably low (1-2%). However, in the developing countries where infections involving the nervous system are common, routine CT scan of brain may help in finding treatable causes of seizures.

Objective:

Aim of this study was to evaluate the significance of CT scan of brain in the management of children with partial seizures.

Materials and Methods:

Children with partial epilepsy, whose predominant seizure type was focal motor seizures, were included in the study. CT scan of brain was done in all children aged between 1 month and 12 years with partial seizures of unknown etiology prospectively. The clinical findings of these children were noted along with the CT findings.

Results:

Between August 2001 and July 2002, of the 200 children with seizure disorder 50 children who satisfied the inclusion criteria were included in the study. CT scan of brain was normal in 16 children (32%) and was abnormal in 34 children (68%). Twenty children (~60% of abnormal scan) had potentially correctable lesions: Tuberculoma (n = 13), neurocysticercosis (n = 3), and brain abscess (n = 4). Five children had changes representing static pathology that did not influence patient management. The clinical features correlated with CT findings in 78% children.

Conclusion:

Children with partial motor seizures have high probability of having abnormal findings on CT scan of brain, especially, neuro-infections which are potentially treatable. Therefore, CT scan brain should be carried out in all children with partial motor seizures especially, in developing countries.     

Idiopathic generalized epilepsy: Phenotypic and electroencephalographic observations in a large cohort from South India

Purpose:

We studied the phenotype and electroencephalographic (EEG) features, and therapeutic aspects of idiopathic generalized epilepsies (IGEs) in South Indian population.

Patients and Methods:

This prospective cross-sectional hospital-based study was carried out on non-consecutive 287 patients (age 22.2 ± 7.7 years; M:F = 139:148) with IGE syndrome. Their clinical and EEG observations were analyzed.

Results:

Majority of the patients had onset of seizures <20 years of age (n = 178; 62%). Thirty one patients (10.8%) had family history of epilepsy. Nearly half of them (49.9%) had <5 years of duration of seizures. The type of IGEs included Juvenile myoclonic epilepsy (JME): 115 (40.1%); IGE with generalized tonic-clonic seizures (GTCS) only: 102 (39.02%); childhood absence epilepsy (CAE): 35 (12.2%); GTCS on awakening: 15 (5.2%); Juvenile absence epilepsy (JAE): 11 (3.8%); and unclassified seizures: 9 (3.1%). The triggering factors noted in 45% were sleep deprivation (20%), non-compliance and stress in 5% each. The EEG (n = 280) showed epileptiform discharges in about 50% of patients. Epileptiform discharges during activation was observed in 40/249 patients (16.1%): Hyperventilation in 32 (12.8%) and photic stimulation in 19 (7.6%). The seizures were well controlled with anti-epileptic drugs (AEDs) in 232 (80.8%) patients and among them, 225 (78.4%) patients were on monotherapy. Valproate (n = 131) was the most frequently prescribed as monotherapy.

Conclusions:

This is one of the largest cohort of patients with IGE. This study reiterates the importance of segregating IGE syndrome and such analysis will aid to the current understanding and management.     

Clinical profile of patients with nascent alcohol related seizures

Aim:

The aim of this study is to characterize the clinical profile of patients with alcohol related seizures (ARS) and to identify the prevalence of idiopathic generalized epilepsy (IGE) in the same.

Materials and Methods:

100 consecutive male patients presenting to a tertiary care center in South India with new onset ARS were analyzed with alcohol use disorders identification test (AUDIT) score. All underwent 19 channel digital scalp electroencephalography (EEG) and at least computed tomography (CT) scan.

Results:

A total of 27 patients (27%) who had cortical atrophy on CT had a mean duration of alcohol intake of 23.62 years compared with 14.55 years in patients with no cortical atrophy (P < 0.001). Twenty-two patients (22%) had clustering in the current episode of whom 18 had cortical atrophy. Nearly, 88% patients had generalized tonic clonic seizures while 12% who had partial seizures underwent magnetic resonance imaging (MRI), which identified frontal focal cortical dysplasia in one. Mean lifetime duration of alcohol intake in patients presenting with seizures within 6 hours (6H-gp) of intake of alcohol was significantly lower (P = 0.029). One patient in the 6H-gp with no withdrawal symptoms had EEG evidence for IGE and had a lower AUDIT score compared with the rest.

Conclusion:

CT evidence of cortical atrophy is related to the duration of alcohol intake and portends an increased risk for clustering. Partial seizures can be a presenting feature of ARS and those patients may benefit from MRI to identify underlying symptomatic localization related epilepsy (8.3% of partial seizures). IGE is more likely in patients presenting with ARS within first 6 hours especially if they do not have alcohol withdrawal symptoms and scalp EEG is helpful to identify this small subgroup (~1%) who may require long-term anti-epileptic medication.Key Words: Alcohol use disorders identification test, anti-epileptic drugs, convulsions, electroencephalography, ethanol, magnetic resonance imaging     

Neuroimaging observations in a cohort of elderly manifesting with new onset seizures: Experience from a university hospital

Background:

The occurrence of epilepsy is higher among elderly patients. The clinical manifestations of seizures, causes of epilepsy, and choice of anti-epileptic drugs (AEDs) are different in elderly people with epilepsy compared to the young.

Aim:

To evaluate the imaging (CT/MRI) observations in elderly patients manifesting with new-onset seizures.

Materials and Methods:

Two hundred and one elderly patients with new onset seizures, >60 years (age: 68.0 ± 7.5 years; M:F = 1.8:1) from Jan’ 07 to Jan’ 09, were prospectively recruited. Observations of cranial CT scan (n = 201) and MR imaging (n = 43) were analyzed.

Results:

The type of seizures included: Simple partial (42%), generalized tonic-clonic (30.3%), and complex partial (27.4%). The pattern of epilepsy syndromes were acute symptomatic (42.3%), remote symptomatic (18.4%), cryptogenic (37.8%), and idiopathic (1.5%). Seizures were controlled with monotherapy in 85%. The CT scan (n = 201) revealed cerebral atrophy (139), mild (79), moderate (43), and severe (18); focal lesions (98), infarcts (45), hemorrhages (18), granuloma (16), tumor (15) and gliosis (4), and hemispheric atrophy (1), white matter changes (75) and diffuse edema (21). An MRI (n = 43) showed variable degree of cerebral atrophy (31); white matter changes (20); focal cerebral lesions (24); - infarct (7); intracranial hemorrhage (6); granuloma (5); tumor (6); gliosis (1); hemispheric atrophy (1); and prominent Virchow-Robin spaces (7); and UBOs (12). Patients with focal lesions in neuroimaging more often had partial seizures, symptomatic epilepsy, past stroke, focal deficit, absence of diffuse atrophy, focal EEG slowing, abnormal CSF, seizure recurrence at follow-up (P < 0.05).

Conclusions:

Brain imaging observations in elderly patients with new-onset seizures revealed underlying symptomatic nature, hence the etiology and thereby assisted in deciding the specific therapy.     

Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy

Context:

Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients.

Aim:

To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy.

Materials and Methods:

A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed.

Results:

Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel''s class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal.

Conclusions:

Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions.     

Seizures in patients with cerebral hemiatrophy: A prognostic evaluation

Purpose:

Cerebral hemiatrophy is a common childhood disease. It clinically manifests with seizures, hemiparesis and mental retardation.

Materials and Methods:

In this prospective study, previously untreated patients with seizures and cerebral hemiatrophy were recruited. Cerebral hemiatrophy was diagnosed on the basis of hemispheric ratio. Patients with acquired hemiconvulsion, hemiplegia, and epilepsy (HHE) syndrome were included in group A. Group B included patients with congenital HHE syndrome. Patients were followed up for 6 months for seizure recurrence.

Results:

Out of 42 patients 26 were in group A and 16 were in group B. After 6 months, there was significant reduction in seizure frequency (P < 0.0001) in both the groups. At least 50% reduction in seizure frequency was noted in all the patients. Complete seizure freedom was observed in 15 (35.7%) patients. Seizure recurrences were significantly higher (P = 0.008) in group A. On univariate analysis, predictors of seizure recurrences were history of febrile seizures (P = 0.013), hippocampal sclerosis (P = 0.001), thalamic atrophy (P = 0.001), basal ganglia atrophy (P = 0.001), cerebellar atrophy (P = 0.01), ventricular dilatation (P = 0.001), epileptiform discharges at presentation (P = 0.023), complex partial seizures (P = 0.006) and status epilepticus (P = 0.02). On multivariate analysis, hemispheric ratio was the only significant factor for seizure recurrence.

Conclusion:

Patients with congenital hemiatrophy had better seizure control than that in patients with HHE syndrome.     

Pattern of seizure cases in tertiary care hospitals in Karnataka state of India

Background:

The prevalence and incidence of epilepsy is higher in developing countries than in developed countries. Understanding pattern and risk factors of seizure cases will help in suggesting appropriate preventive measures.

Objectives:

This study was carried out to assess the pattern of seizure, its management and compliance with treatment.

Materials and Methods:

Data from medical records of seizure cases in three tertiary care hospitals of Mangalore city in south India admitted from January 2006 to December 2011 were collected and analyzed.

Results:

Nearly half (44.4%) of the 196 cases belonged to productive age group (15-45 years) and 2/3^rd (60.7%) were males. Majority (>80% cases) were unskilled workers and of low socio-economic status groups. Family history of seizures was present in 8.4% cases. Mean age of onset of seizure was found to be 19.9 years. Proportion of generalized tonic clonic seizure cases was 78.1%. Secondary seizures were seen in 66 (33.7%) cases with the most common cause being trauma to the head (24.2%). Refractory seizures were present in 2.7% cases. Monotherapy was the most commonly followed treatment regimen and phenytoin was the most popular anti-epileptic drug (AED) used. Non-compliance with AEDs was seen in 18.1% cases and was more among patients on polytherapy (P = 0.032).

Conclusion:

Seizure manifestations and treatment compliance vary widely in the studied population. In depth analysis of each seizure type will give more information about the factors associated with it.     

Termination patterns of stimulus-induced rhythmic,periodic, or ictal patterns and spontaneous electrographic seizures

Objective

To investigate the ability of the evolution and termination patterns to distinguish stimulus-induced rhythmic, periodic, or ictal discharges (SIRPIDs) from spontaneous electrographic seizures, a challenge to the neurophysiologist and clinician.

Optimizing the management of neuromyelitis optica and spectrum disorders in resource poor settings: Experience from the Mangalore demyelinating disease registry

Background:

In resource-poor settings, the management of neuromyelitis optica (NMO) and NMO spectrum (NMOS) disorders is limited because of delayed diagnosis and financial constraints.

Aim:

To device a cost-effective strategy for the management of NMO and related disorders in India.

Materials and Methods:

A cost-effective and disease-specific protocol was used for evaluating the course and treatment outcome of 70 consecutive patients.

Results:

Forty-five patients (65%) had a relapse from the onset and included NMO (n = 20), recurrent transverse myelitis (RTM; n = 10), and recurrent optic neuritis (ROPN; n = 15). In 38 (84.4%) patients presenting after multiple attacks, the diagnosis was made clinically. Only 7 patients with a relapsing course were seen at the onset and included ROPN (n = 5), NMO (n = 1), and RTM (n = 1). They had a second attack after a median interval of 1 ± 0.9 years, which was captured through our dedicated review process. Twenty-five patients had isolated longitudinally extensive transverse myelitis (LETM), of which 20 (80%) remained ambulant at follow-up of 3 ± 1.9 years. Twelve patients (17%) with median expanded disability status scale (EDSS) of 8.5 at entry had a fatal outcome. Serum NMO-IgG testing was done in selected patients, and it was positive in 7 of 18 patients (39%). Irrespective of the NMO-IgG status, the treatment compliant patients (44.4%) showed significant improvement in EDSS (P ≤ 0.001).

Conclusions:

Early clinical diagnosis and treatment compliance were important for good outcome. Isolated LETM was most likely a post-infectious demyelinating disorder in our set-up. NMO and NMOS disorders contributed to 14.9% (45/303) of all demyelinating disorders in our registry.Key Words: Demyelinating disease registry, immunosuppression, India, neuromyelitis optica, neuromyleitis optica spectrum disorders     

Factors predicting surgical outcome of thymectomy in myasthenia gravis: A 16-year experience

Aim:

To assess the surgical outcome of myasthenia gravis (MG) following thymectomy and to determine the outcome predictors to such therapeutic approach.

Materials and Methods:

This study is a retrospective review of 80 consecutive thymectomies performed for MG over a 16-year period.

Results:

There were 41 females and 39 males (mean age, 34.32 years) with mean disease duration of 17.45 months prior to surgery. Stagewise distribution of the patients revealed 2.5% in stage I, 48.7% in stage IIA, 33.8% in stage IIB, 8.7% in stage III, and 6.3% in stage IV. The surgical approach was either trans-sternal (n=67) or video-assisted thoracoscopic route (n=13). Follow-up was obtained in 91.2% (n=73) of patients with mean duration of 67.7 months. At their last follow-up, 26.0% were in complete remission, 35.6% were asymptomatic on decreased medications, and 17.8% had clinical improvement on decreased medications. Overall, 79.4% of patients benefited from surgery, 8.2% had unchanged disease status, and 12.3% worsened clinically. Factors influencing favorable outcome include sex, disease stage, gland weight, and preoperative medication with anti-cholinesterase (P<0.05). There was one death in the perioperative period due to septicemia. Two patients died at fourth and seventh month following thymectomy.

Conclusion:

Thymectomy for MG is safe and effective. Certain influencing factors may shape treatment decisions and target higher risk patients.     

Autoimmune encephalitis: A potentially reversible cause of status epilepticus,epilepsy, and cognitive decline

Objectives:

To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline.

Design:

Observational, prospective case series.

Setting:

All India Institute of Medical Sciences, New Delhi, India.

Materials and Methods:

Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor''s information) were noted.

Results:

There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation.

Conclusions:

Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis.Key Words: Autoimmune encephalitis, cognitive decline, drug refractory epilepsy, seizures, status epilepticus     

Bilateral independent periodic discharges are associated with electrographic seizures and poor outcome: A case-control study

Objective

To determine the clinical correlates bilateral independent periodic discharges (BIPDs) and their association with electrographic seizures and outcome.

Severe myoclonic epilepsy of infancy (Dravet syndrome): Clinical and genetic features of nine Turkish patients

Purpose:

Mutations of the α-1 subunit sodium channel gene (SCN1A) cause severe myoclonic epilepsy of infancy (SMEI). To date, over 300 mutations related to SMEI have been described. In the present study, we report new SCN1A mutations and the clinical features of SMEI cases.

Materials and Methods:

We studied the clinical and genetic features of nine patients diagnosed with SMEI at the Pediatric Neurology Department of Istanbul Medical Faculty.

Results:

Five patients had nonsense mutations, two had missense mutations, one had a splice site mutation and one had a deletion mutation of the SCN1A gene. Mutations at c.3705+5G splice site, p.trip153X nonsense mutation and deletion at c.2416_2946 have not been previously described. The seizures started following whole cell pertussis vaccination in all patients. The seizures ceased in one patient and continued in the other eight patients. Developmental regression was severe in three patients, with frequent status epilepticus. The type of mutation was not predictive for the severity of the disease. Two of the three patients with severe regression had nonsense and missense mutations.

Conclusions:

Dravet syndrome can be result of several different types of mutation in SCN1A gene. Onset of the seizures after pertussis vaccination is an important clue for the diagnosis and neuro- developmental delay should be expected in all patients.     

Clinical and psychosocial characteristics of children with nonepileptic seizures

Objective:

The aim of this study is to present a comprehensive profile of clinical and psychosocial characteristics of children with psychogenic nonepileptic seizures and to assess the short-term outcome of these patients.

Materials and Methods:

The subjects were consecutive cases of children with a diagnosis of nonepileptic seizures (N=17, mean age = 10.7 years, S.D. = 1.26) and two groups of control groups matched on age and sex: true seizure group and healthy controls. All the children were recruited from the out-patient services of the Department of Pediatrics of a tertiary care teaching hospital in North India. Detailed history taking and clinical examination was done in the case of every child. A standard 18 channel EEG was done in all the children and a video EEG was done in 12 cases of children with nonepileptic seizures. The Childhood Psychopathology Measurement Schedule (CPMS) and Life Events Scale for Indian Children (LESIC) were used to measure the children''s emotional and behavioral functioning at home, and the number of life events and the stress associated with these events in the preceding year and the year before that. Short-term outcome was examined three to six months after the diagnosis of nonepileptic seizures was made.

Results:

Unresponsiveness without marked motor manifestations was the most common “ictal” characteristic of the nonepileptic seizures. Pelvic thrusting, upper and lower limb movements, head movements, and vocalization were observed in less than one-third of the patients. Increased psychosocial stress and significantly higher number of life events in the preceding year were found to characterize children with nonepileptic seizures, as compared to the two control groups. The nonepileptic seizures and true seizures groups had a higher proportion of children with psychopathology scores in the clinically significant maladjustment range, as compared to those in the healthy control group. A majority of the patients (82.4%) either recovered completely or had more than 50% reduction in the frequency of their symptoms, after three to six months of initiation of therapy.

Conclusions:

Psychosocial stress is common among children with nonepileptic seizures. Confirmatory diagnosis by video EEG, along with prompt psychosocial intervention, often results in a favorable outcome for most children with nonepileptic seizures.